Hemostatic rebalance in Neonatal intrahepatic cholestasis wi
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Neonatal intrahepatic cholestasis with citrin deficiency (NICCD) results in coagulopathy due to decreased levels of vitamin (V)K dependent clotting factors, similar to biliary atresia (BA).

A Study was conducted to examine the relationships between coagulant and anticoagulant potential, with addition to the influence of nutritional treatment, in NICCD.

Three cases (aged 12, 21, and 45 days) with NICCD associated coagulopathy were evaluated with standard coagulation/anticoagulation tests and comprehensive coagulation assays, rotational thromboelastometry (ROTEM), and protein C/protein S (PC/PS) pathway function assay (ThromboPath®), before and after nutritional treatment.

--In all cases, activated partial thromboplastin time and prothrombin time were significantly prolonged associated with very low levels of VK independent fibrinogen and antithrombin.

--The initiation of nutritional treatment of medium chain triglycerides oil had improved these levels within normal range, although low levels of other clotting factors were modestly increased.

--Whole blood?ROTEM analysis revealed near normal coagulation potentials even before treatment, comparable to the healthy adults, supportive of their non-bleeding symptoms.

--The introduction of nutritional treatment had further improved comprehensive coagulation potentials.

--The global PC/PS pathway function assay demonstrated the absence of the features of this function associated with the pathogenesis of NICCD.

--Compared to BA, plasma levels of fibrinogen and antithrombin in all cases were markedly low, whilst those after treatments improved, especially to similar level in fibrinogen.

Finally, NICCD has a rebalancing hemostatic pathway with coagulant and anticoagulant potential involving low levels of fibrinogen and antithrombin, implying a pathophysiological coagulopathy distinct from BA.

Source: https://onlinelibrary.wiley.com/doi/10.1111/ped.14741?af=R